Abstract: Objective To explore the clinicopathological features of angiocentric glioma. Methods The clinical manifestations, radiologic and histopathological features and immunohistochemical results were analyzed in one case of angiocentric glioma, with reviewing of the related literatures. Results The patient was a 12-year-old girl with a 2-year history of instability of right hand and 1-year history of onset of syncope associated with twitching limbs. Magnetic resonance imaging (MRI) examination showed T1-hypointense and T2-hyperintense lesions in the left temporal-parietal lobe without obvious enhancement. Morphologically, the tumor was of diffuse growth pattern, with no boundary with normal brain tissue. The tumor was formed by monomorphous bipolar spindle cells arranged in fascicles and pseudorosettes surrounding blood vessels. Tumor cells were well-differentiated, but showed an infiltrating biological behavior. No mitosis was apparent. Microvascular proliferation and necrosis were absent. Immunohistochemically, tumor cells were reactive for glial fibrillary acidic protein (GFAP), nestin and EMA, and the latter showed an characteristic 'dot-like' pattern. Tumor cells were negative for cytokeratin (CK), E-cadherin, synaptophysin (Syn), neurofilament (NF), NeuN and P53. Ki-67 labelling index was less than 1%. Conclusion Angiocentric glioma has both the features of ependymoma and diffuse astrocytoma. Its diagnosis mainly depends on histopathologic analysis and immunohistochemical staining.

Key words: Glioma, Pathology, Immunohistochemistry

摘要： 目的 探讨血管中心型胶质瘤的临床表现及病理特征。方法 对1 例血管中心型胶质瘤患者的临床表现、影像学特点、组织形态学和免疫表型进行分析，并结合文献探讨其诊断与鉴别诊断要点。结果 女性患者，12 岁。主诉右手持物不稳2 年，发作性晕厥伴四肢抽搐1 年。头部MRI 显示左侧颞顶叶交界区边界不清、椭圆形病变，无明显占位效应，T1WI呈等或稍低信号、T2WI呈高信号，未见明显强化。术中可见肿瘤组织呈灰白色、血运较为丰富、质地柔软，分块切除。光学显微镜观察肿瘤细胞呈弥漫性生长，与周围正常脑组织无明显界限，主要由形态单一的双极细胞围绕血管生长，部分呈放射状排列并形成血管周围假“菊形”团结构，肿瘤细胞分化良好但呈侵袭性生长，侵犯脑实质及软脑膜；无或极少见核分裂象，无微血管增生和坏死。肿瘤细胞胶质纤维酸性蛋白、巢蛋白表达呈弥漫强阳性；上皮膜抗原呈特征性点状阳性；细胞角蛋白、E-钙黏蛋白、突触素、神经微丝、神经元核抗原、P53 表达阴性；Ki-67 抗原标记指数为1%。结论 血管中心型胶质瘤兼具室管膜瘤和弥漫性星形细胞瘤的特点，其诊断和鉴别诊断主要依靠组织病理学和免疫组织化学检测。

关键词: 神经胶质瘤, 病理学, 免疫组织化学